Introduction
Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is scarce and usually presents as meningoencephalomyelitis. Here, we offer the case of an atypical presentation of ...GFAP-astrocytopathy.
Case Presentation
We report the case of a 26-year-old woman admitted to our neurology department for a 3-week progressive and worsening neurologic picture, with secondary worsening. Initial imaging showed a Mild Encephalitis with Reversible Splenium of corpus callosum lesion (MERS). Full infectious and autoimmune workup then revealed positivity of GFAP antibodies, leading us to diagnose GFAP astrocytopathy.
Discussion
Our case is the first reported association between MERS and GFAP astrocytopathy in an adult patient. Clinical presentation of GFAP astrocytopathy usually includes various neurologic symptoms and can lead to misdiagnosis.
INTRODUCTIONRituximab (RTX) has been proven effective in managing refractory generalized myasthenia gravis (MG), and its use is increasing worldwide. MG stabilization may initially require oral ...corticosteroid (CS) therapy, but its long-term side effects require the shortest duration of treatment. We studied the clinical effectiveness and usefulness of corticosteroids associated with RTX compared to RTX alone on MG remission.METHODSIn a monocentric retrospective cohort in the Nice University Hospital, we compared naïve MG patients treated with RTX as first-line therapy alone (G1) or associated with CS (G2). After the RTX induction, we evaluated efficacy with the Osserman score (OS) and the requirement for any rescue therapy (IVIg or plasmapheresis).RESULTSSixty-eight patients were treated with RTX, of which 19 (27.94%) benefited from an association with at least 0.5 mg/kg of corticosteroids. RTX-CS patients were more severe than RTX alone (OS for G1: 74.1 and G2: 64.94, p = 0.044). However, OS at 3 (83.44 and 83.12, p = 0.993), 6 (88.69 and 86.36, p = 0.545), 9 (82.91 and 85.73, p = 0.563), and 12 months (86.6 and 88.69, p = 0.761) from the treatment induction were similar. Rescue therapy following RTX induction was significantly higher for the RTX-CS (20.41% and 47.37%, p = 0.037). Regarding safety, adverse event rates were similar in the two groups (0% and 14.29%, p = 0.178).CONCLUSIONWe suggest that RTX alone is as effective as RTX-CS in MG patients, indicating that avoiding steroids could reduce side effects, decrease rescue therapies, and not affect MG outcomes.
The diagnosis and management of inflammatory bowel disease relies on histological assessment, which is costly, subjective, and lacks utility for point-of-care diagnosis. Fourier-transform infra-red ...spectroscopy provides rapid, non-destructive, reproducible, and automatable label-free biochemical imaging of tissue for diagnostic purposes. This study characterises colitis using spectroscopy, discriminates colitis from healthy tissue, and classifies inflammation severity. Hyperspectral images were obtained from fixed intestinal sections of a murine colitis model treated with cell therapy to improve inflammation. Multivariate analyses and classification modelling were performed using supervised and unsupervised machine-learning algorithms. Quantitative analysis of severe colitis showed increased protein, collagen, and nucleic acids, but reduced glycogen when compared with normal tissue. A partial least squares discriminant analysis model, including spectra from all intestinal layers, classified normal colon and severe colitis with a sensitivity of 91.4% and a specificity of 93.3%. Colitis severity was classified by a stacked ensemble model yielding an average area under the receiver operating characteristic curve of 0.95, 0.88, 0.79, and 0.85 for controls, mild, moderate, and severe colitis, respectively. Infra-red spectroscopy can detect unique biochemical features of intestinal inflammation and accurately classify normal and inflamed tissue and quantify the severity of inflammation. This is a promising alternative to histological assessment.
The mechanisms affecting epidermal homeostasis during aging remain poorly understood. To identify age-related microRNAs, a class of non-coding RNAs known to play a key role in the regulation of ...epidermal homeostasis, an exhaustive miRNA expression screen was performed in human keratinocytes from young or elderly subjects. Many microRNAs modulated by aging were identified, including miR-30a, in which both strands were overexpressed in aged cells and epidermal tissue. Stable MiR-30a over-expression strongly impaired epidermal differentiation, inducing severe barrier function defects in an organotypic culture model. A significant increase was also observed in the level of apoptotic cells in epidermis over-expressing miR-30a. Several gene targets of miR-30a were identified in keratinocytes, including
(encoding lysyl oxidase, a regulator of the proliferation/differentiation balance of keratinocytes),
(encoding isocitrate dehydrogenase, an enzyme of cellular metabolism) and
(encoding a caspase inhibitor). Direct regulation of
,
and
by miR-30a was confirmed in human keratinocytes. They were, moreover, observed to be repressed in aged skin, suggesting a possible link between miR-30a induction and skin-aging phenotype. This study revealed a new miRNA actor and deciphered new molecular mechanisms to explain certain alterations observed in epidermis during aging and especially those concerning keratinocyte differentiation and apoptosis.
The context of the outbreak Since 2004, the number of measles cases reported in Madagascar had plummeted from tens of thousands of annual cases to fewer than 20 confirmed cases per year.4 This drop ...in cases followed a successful expansion of the immunisation programme (figure 1A) in which consistent (although low) routine measles vaccination coverage (between 55% and 85%) was combined with vaccination campaigns targeting ages from 9 months to 14 years (in 2004) or 9 months to 4 years (in 2007, 2010, 2013, 2016).5 The combination of low incidence and the potential for incomplete vaccination coverage over a number of years suggested that Madagascar might have a large susceptible population distributed across a wide age range, yielding potential for a wide age range, fast-growing outbreak. Historical context of measles in Madagascar and the recent outbreak (A) measles first dose vaccination coverage estimates from UNICEF (y-axis, top) and measles incidence from who (y-axis, log scale, bottom) from 1980 to 2018 (x-axis), showing a sharp decline in cases in 2005 with 10 or less cases in each years following 2005 until the start of the focal outbreak in 2018 when more than 21 000 cases reported; (B) time series of suspected cases (y-axis) against time (indicated epidemic week starting in 2018, thin vertical line shows the separation between 2018 and 2019) from the 2018 to 2019 line-list data in each of the 22 regions, coloured by the timing of the peaks (orange is earliest, grey intermediate, and brown latest); dashed vertical lines indicate the approximate timing of the three waves of vaccination (different districts were targeted in each wave, see text); inset shows the regions of Madagascar coloured as for the time series (orange is the earliest peaks, grey intermediate and brown the latest). (A) Measles vaccination coverage at 5 years in each of the 22 regions estimated from Madagascar’s 2009 Demographic and Health Survey (x-axis) plotted against (A) the median age distribution of cases (y-axis) and (B) the R E of the outbreak in each region (y-axis). The outbreak response In October 2018, rapidly growing case numbers in the Analamanga region (where the capital city of Antananarivo is located) prompted deployment of a vaccination campaign across a limited spatial extent (four health districts of the urban community of Antananarivo) targeting children up to 5 years of age.
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